CNS Tumors

What are the classifications of brain tumors

Primary vs Secondary

What are the malignancy categories of brain tumors

“Benign” vs Malignant

How many people in the US per year are diagnosed with a primary brain tumor

~80,000 people in the US per year diagnosed with a primary brain tumor

How many people per year are diagnosed with brain metastases

~200,000 people per year diagnosed with brain metastases

What is the underlying cause for the majority of primary brain tumors

No underlying cause for the majority

What proportion of people with primary brain tumors have a positive family history

Small minority of people have a +FHx

What are some primary brain tumors associated with

genetic syndromes and CNS tumor syndromes (ex. neurophakomatoses)

What are examples of neurophakomatoses

Ex Neurofibromatosis, Tuberous Sclerosis, etc

What are the risk factors for primary brain tumors

Exposure to ionizing radiation, Immunosuppression (primary CNS lymphomas)

What are the symptoms of a primary brain tumor

HA, N/V, cognitive/personality changes, seizures, focal findings, cranial nerve deficits, gait d/o, tumors or edema, which can cause increased ICP or obstructive hydrocephalus.

When are headaches in primary brain tumors usually worst

worse in AM or wake patient from sleep, also may be positional.

What are seizures like in primary brain tumors

a focal onset which may or may not be obvious clinically.

What are the focal findings in primary brain tumors

hemiparesis, sensory deficits, aphasia, visual field defect, vision loss.

What are the red and orange flag s/sxs on the SNNOOP10 for secondary HAs

systemic sxs including fever, neoplasm hx, neurologic deficit or dysfxn (including decreased conciousness), onset of HA is sudden or abrupt, older age (>50), pattern change or recent onset of HA, positional HA, precipitated by sneezing, coughing, or exercise, papilledema, progressive HA and atypical presentations, pregnancy or puerperium, painful eye with autonomic features.

What may neurologic deficits indicate in SNNOOP10

HAs attributed to vascular, nonvascular intracranial d/o

What do systemic symptoms including fever indicate in SNNOOP10

HA attributed to infection or nonvascular intracranial d/o, carcinoid or pheochromocytoma.

What does a history of neoplasms indicate in SNNOOP10

neoplasms of the brain or metastasis.

What is the only symptom in SNNOOP10 that can be orange

systemic symptoms including fever can be orange, when the fever is isolated.

What is the first imaging modality for brain tumor diagnosis

Stat CT brain first imaging modality

What is the preferred imaging for brain tumors

Brain MRI with gad is preferred imaging

What imaging characteristics do malignant tumors typically have on MRI

typically enhance, have central necrosis, and are surrounded by edema.

What is the definitive diagnosis of brain tumors

Biopsy/ Histopathology

What is included in metastatic workup for brain tumors

ex CT chest, abdomen, pelvis

What is the most common malignant primary brain tumor

gliomas.

What are the types of gliomas

Astrocytomas, Oligodendrogliomas, Ependymomas

What are characteristics of astrocytomas

Can be encapsulated or infiltrative tumors.

What are the grades of astrocytomas

Grades I/II considered low grade

What is the grade of oligodendrogliomas

grade II tumors.

What are characteristics of oligodendrogliomas

Less infiltrative than astrocytomas.

What molecular markers subdivide gliomas

Isocitrate-dehydrogenase (IDH) and 1p/19q co-deletion

How are astrocytomas diagnosed molecularly

Astrocytomas are now diagnosed solely by IDH mutations

What is the molecular status of glioblastomas

Glioblastomas are IDH-wildtype tumors

What molecular features define oligodendrogliomas

IDH mutations and 1p/19q co-deletions

What is one of the main types of astrocytomas

glioblastomas.

What type of astrocytoma tumors are more common in children

Low-grade tumors are more common

What is an example of a grade I astrocytoma

Ex pilocytic astrocytoma is grade I

What type of astrocytoma tumors are more common in adults

High-grade tumors are more common

What is the WHO grade of pilocytic astrocytoma

WHO grade 1

What is the growth pattern of pilocytic astrocytoma

Slow-growing, well-circumscribed glial tumor

Where is pilocytic astrocytoma usually seen

Usually seen in children in posterior fossa (cerebellum)

What are the signs and symptoms of pilocytic astrocytoma

ataxia, high ICP

What are the MRI findings of pilocytic astrocytoma

cystic lesion with an enhancing mural nodule

What are the histology findings of pilocytic astrocytoma

Rosenthal fibers

What is the treatment for pilocytic astrocytoma

surgical resection (often curative)

What is the classification of glioblastoma

IDH-wildtype, WHO grade 4

What is the most common malignant primary brain tumor

Most common malignant primary brain tumor

How many glioblastoma cases occur per year in the US

>12,000 cases per year in the US

What characterizes primary glioblastoma

Most common

What characterizes secondary glioblastoma

malignant transformation from low grade astro

What are the presenting symptoms of glioblastoma

HA, sz, or focal symptoms

What is the growth behavior of primary glioblastomas

Primary are fast growing and aggressive

What is the infiltrative behavior of glioblastoma

Highly infiltrative

What is the prognosis of glioblastoma

Poor prognosis

What is the 5-year survival rate of glioblastoma

5-year survival 10%

What histologic features are seen in glioblastoma

high-grade features such as microvascular proliferation or necrosis

What MRI findings are seen in glioblastoma

enhancing mass with central necrosis and surrounding edema

What structure may glioblastoma cross

corpus callosum (butterfly glioma)

What are the histology findings of glioblastoma

malignant astrocytes, necrotizing, hemorrhagic center surrounded by pleomorphic cells in a pseudopalisading pattern (tumor cells lining the area of necrosis)

What is the treatment for glioblastoma

resection, radiation, chemo (temozolomide)

What additional treatment may be used in glioblastoma

may implant biodegradable polymers containing chemo

What is the prognosis of glioblastoma despite optimal treatment

tumor recurs

What percentage of gliomas are oligodendrogliomas

15-20% of all gliomas

What grade are oligodendrogliomas

Grade II

In what age group are oligodendrogliomas commonly found

middle-aged adults

What are the MRI findings of oligodendroglioma

non enhancing mass, often partially calcified

What biopsy findings are seen in oligodendroglioma

1p/19q co-deletion and IDH mutation

What are the histology findings of oligodendroglioma

calcifications, hemorrhage or cystic areas

What is the treatment for oligodendroglioma

surgery, +/- radiation and chemo, but more responsive to therapies.

From what cells are ependymomas derived

cells that line the ventricular surface and central canal

In what age groups are ependymomas most common

young children and young adults

Where are ependymomas most often located in children

Most are infratentorial

What symptoms may infants with ependymoma present with

hydrocephalus (increased head size can be seen), irritability, depressed mental status, vomiting

What symptoms may older children with ependymoma present with

headache, n/v

Where are ependymomas more common in adults

the spine, esp the filum terminale of the spinal cord

What may adults with spinal ependymomas present with

cauda equina

What are the MRI findings of ependymoma

hypointense T1

What are the histology findings of ependymoma

perivascular pseudorosettes

What is the treatment for ependymoma

surg resection

What is the prognosis of ependymoma if fully resected

Potentially curable if fully resected, although there are rare aggressive subtypes

What is the most common primary brain tumor

meningioma

What factors increase the risk of meningioma

Increase with age, more common in women (tumor cells have estrogen receptors), assoc with NF 2, more common with hx cranial irradiation

How are meningiomas often found

Often found incidentally on neuroimaging

How can meningiomas become symptomatic

Can be symptomatic due to compression and displacement of brain

What is the growth pattern of meningiomas

Usually benign and slow-growing

From where do meningiomas most often arise

Most often arises from the dura

What are the grades of meningiomas

Grade I (benign) to Grade III (malignant)

What are the MRI findings of meningioma

Dural based enhancing tumors with a dural tail and compression of brain

What are the histology findings of meningioma

spindle cells concentrically arranged in a whorled pattern

What is the treatment for asymptomatic small meningioma

observation if small

What is the treatment for symptomatic or large meningioma

surgical excision

What type of tumor is schwannoma, and what does it arise from

Benign

What is the most common type of schwannoma

Vestibular schwannomas (aka acoustic neuromas) are most common

What percentage of primary brain tumors are schwannomas

9% of primary brain tumors

What condition increases the risk of schwannoma

Increased risk with neurofibromatosis

What symptoms may schwannomas cause

Asymptomatic or unilateral hearing loss, dizziness, tinnitus (less commonly brainstem or cerebellar symptoms)

What are the MRI findings of schwannoma

densely enhancing lesions, enlarging internal auditory canal, often extending into the cerebellopontine angle

What is the treatment for very small and asymptomatic schwannomas

can observe with serial MRIs

What is the treatment for larger or symptomatic schwannomas

surgery or stereotactic radiosurgery

What increases the chance of preserving hearing in schwannoma

Patients with small ones and intact hearing, early surgical intervention increases the chance of preserving hearing

What percentage of primary brain tumors are pituitary adenomas

Up to 10% of all primary brain tumors